Kasabach-Merritt syndrome, also known as haemangioma thrombocytopaenia syndrome, is a rare life-threatening disease found in infants in which a rapidly growing vascular tumour is responsible for thrombocytopaenia, microangiopathic haemolytic anaemia and consumptive coagulopathy.
Vascular tumours may grow very rapidly, which can induce severe thrombocytopenia and severe consumptive coagulopathy. In severe cases, there may be disseminated intravascular coagulation.
The radiographic features are specific to each vascular tumour. They can be found in multiple locations, more frequently subcutaneous, musculoskeletal, splenic and hepatic in location.
Treatment and prognosis
Various treatments are used, with varying degrees of success, in Kasabach-Merritt syndrome - systemic corticosteroids, radiation therapy, compression, embolisation and/or pharmacotherapy (e.g. interferon, platelet aggregation inhibitors) 4.
Repetitive haemarthrosis can also occur, leading to a haemophilia-like arthropathy 1. Other complications include congestive heart failure and gastrointestinal bleeding 3.
History and etymology
The syndrome is named after Haig Haigouni Kasabach and Katharine Krom Merritt, the two paediatricians who first described the condition in 1940.
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- 3. Elsayes KM, Menias CO, Dillman JR et-al. Vascular malformation and hemangiomatosis syndromes: spectrum of imaging manifestations. AJR Am J Roentgenol. 2008;190 (5): 1291-9. doi:10.2214/AJR.07.2779 - Pubmed citation
- 4. Hesselmann S, Micke O, Marquardt T et-al. Case report: Kasabach-Merritt syndrome: a review of the therapeutic options and a case report of successful treatment with radiotherapy and interferon alpha. Br J Radiol. 2002;75 (890): 180-4. Br J Radiol (full text) - Pubmed citation